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Review
. 2015:2015:102656.
doi: 10.1155/2015/102656. Epub 2015 Apr 1.

Molecular Pathogenesis of MALT Lymphoma

Katharina Troppan  1 , Kerstin Wenzl  1 , Peter Neumeister  1 , Alexander Deutsch  1
Affiliations
Review

Molecular Pathogenesis of MALT Lymphoma

Katharina Troppan et al. Gastroenterol Res Pract. 2015.

Abstract

Approximately 8% of all non-Hodgkin lymphomas are extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT), also known as MALT lymphoma, which was first described in 1983 by Isaacson and Wright. MALT lymphomas arise at a wide range of different extranodal sites, with the highest frequency in the stomach, followed by lung, ocular adnexa, and thyroid, and with a low percentage in the small intestine. Interestingly, at least 3 different, apparently site-specific, chromosomal translocations and missense and frameshift mutations, all pathway-related genes affecting the NF-κB signal, have been implicated in the development and progression of MALT lymphoma. However, these genetic abnormalities alone are not sufficient for malignant transformation. There is now increasing evidence suggesting that the oncogenic product of translocation cooperates with immunological stimulation in oncogenesis, that is, the association with chronic bacterial infection or autoaggressive process. This review mainly discusses MALT lymphomas in terms of their genetic aberration and association with chronic infections and summarizes recent advances in their molecular pathogenesis.

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Figures

Figure 1
Figure 1
Translocations affecting the NF-κB activation pathway. (a) Signaling from the TLR, IL-1R, and antigen receptor activates the canonical NF-κB pathway, which is characterized by activation of the IKK complex, phosphorylation, and degradation of IκB. TNFAIP3 is a negative regulator. (b) t(1;14)(p22;q32) results in the nuclear overexpression of the BCL10 protein. It is believed to form oligomers through its CARD domain and so it triggers MALT1 oligomerization and aberrant NF-κB activation. (c) t(14;18)(q32;q21) causes overexpression of MALT1. It is thought that it oligomerizes through interaction with BCL10 causing NF-κB activation. (d) t(11;18)(q21;q21), the BIR domain of the BIRC2-MALT1, mediates self-oligomerization leading to an activation of NF-κB. TLR: Toll-like receptor; IL-1R: interleukin-1 receptor; BCR: B cell receptor; TCR: T cell receptor; RIP1: receptor interacting protein 1; TRAF: TNF-associated factor; TAK1: transforming growth factor beta activated kinase 1; TAB: TAK binding protein; IKK: inhibitor of NF-κB kinase; IκB: inhibitor of NF-κB.
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